Wednesday, May 8, 2013

Newsletter - Peutz-Jeghers Syndrome & Juvenile Polyposis Syndrome


OUTDATED!
FOR ARCHIVE ONLY!

May 2013

These resources are especially for those interested in PJS & JPS - the diagnosed, their friends, families, and health professionals. Please share this with any and all. A list of Online Support Groups for people interested in inherited cancer syndromes is at bottom of this email.

Warmest healing wishes,
Stephanie Sugars
pj4steph@aol.com

Jump to Newsletter



*****
INFORMATION AND NEWS

* The fifth Peutz-Jeghers Syndrome Meet & Greet was held on July 27, 2013 in Texas. 

* A PJS information day was held in the Erasmus MC in Rotterdam on 8 June, 2013. 

* The fourth Peutz-Jeghers Syndrome Meet & Greet was held in July 2012 in Pennsylvania.

* The third Peutz-Jeghers Syndrome Meet & Greet was held in Denver, Colorado
July 15, 2011. For a copy of the DVD, please contact Heidi at

* The Summer 2011 issue of Familial Cancer journal focuses on
Peutz-Jeghers syndrome. The issue includes an article by me,
Peutz-Jeghers syndrome: a patient’s view. It can be purchased for
$34.95 at

Or read in an unformatted version at

* Peutz-Jeghers News blog by Stephanie Sugars 

* How to have a PJS-free baby. This is a report that I wrote for the
PJS Online Support Group (http://listserv.acor.org/archives/pjs.html).

* 2012 PJS and family planning: the attitude towards prenatal genetic testing and pre-implantation genetic diagnosis

* Genetic mosaicism has now been described in PJS people. This is something patients and family members should discuss with genetic counselors…but they don't have the information yet.

* 2013 GeneReviews PJS entry
 and  2011 Juvenile Polyposis syndrome entry
 Directories for genetic testing laboratories and clinics can be found at: http://www.genetests.org/

* 2013 eMedicine article on PJS and JPS

* 2013 Genetics Home Reference
Peutz-Jeghers syndrome entry

* 2012 Juvenile polyposis syndrome: a study of genotype, phenotype, and long-term outcome
free slide presentation

* 2011 article about JPS - Juvenile polyposis syndrome

* 2010 article from 2007 conference of European PJS specialists
Peutz–Jeghers syndrome: a systematic review and recommendations for Management
free full text

* 2010 free full text article includes PJS and Juvenile Polyposis
Syndrome - Hereditary hamartomatous polyposis syndromes: understanding the disease risks as children reach adulthood. by M. Manfredi

* 2008 50+ page report from Mayo Clinic on PJS,
appropriate for patients, family, doctors and genetic counselors at
New 2010 addition on PJS in children and adolescents

* 2009 Juvenile Polyposis entry from Cancer Syndromes textbook

* 2009 Juvenile Polyposis syndrome entry from Genetics Home Reference

* 2009 screening guidelines for PJS and Juvenile Polyposis Syndrome
 from National Comprehensive Cancer Network at
(free  registration required).

* 2009 PJS in the news. This story is about mice, not people. And no, these drugs aren't currently available to PJS human patients, though there was a small trial of them in Utah . And the suggested FDG-PET scans for screening have several drawbacks including accuracy, expense & radiation exposure. Still, it's interesting that mouse PJS polyps show increased glucose metabolism. The PJS gene LKB1 affects both metabolism and cancer risk. Press release:  http://www.eurekalert.org/pub_releases/2009-06/si-hc061209.php

* 2007 Medscape article on PJS, JPS & similar polyposis syndromes (free registration required)

* 2006 Frequency and Spectrum of Cancers in the Peutz-Jeghers Syndrome.

* 2006 Peutz-Jeghers Syndrome and Management Recommendations. This
article from Johns Hopkins includes suggested screening for PJS people
& at-risk family members. Click on full-text pdf for free full text.

* There are many new articles on PJS and the PJS gene LKB1 or STK11

TREATMENT OPTIONS
* Learn more about the PillCam capsule endoscopy for small bowel
imaging and find a physician in your region at www.givenimaging.com

* Enteroscopy allows the entire small bowel to be scoped and replaces
major and endoscopic surgery in some PJS patients. Interventional balloon enteroscopy at http://www.ncbi.nlm.nih.gov/pubmed/22586550

* Learn more about double balloon enteroscopy, a new surgical method
and get referrals to doctors who use it at www.fujinonendoscopy.com .
Learn more about its use in adults at

* Olympus has technologies for reaching and visualizing the small bowel including endocapsule, spiral enteroscopy and single balloon enteroscopy. http://www.olympusamerica.com/msg_section/index.asp

GENETIC TESTING
* The new genetic test method, MLPA, has increased the accuracy of genetic testing for PJS. Those with previously negative or inconclusive test results may wish to be retested. Clinic and laboratory directories: http://www.ncbi.nlm.nih.gov/sites/GeneTests/?db=GeneTests

* Genetic testing is available (no travel necessary) through Professor
Rodney Scott, Division of Genetics, Hunter Area Pathology Services,
John Hunter Hospital, Lookout Road, New Lambton, NSW 2305 Australia.
Check with Dr. Scott for cost Rodney.Scott@newcastle.edu.au

TRIALS AND STUDIES SEEKING PARTICIPANTS
* For PJS patients with metastatic cancer, there’s a clinical trial of
everolimus in the Netherlands.
A 2011 article about the use of everolimus in a patient with PJS and pancreatic cancer by the investigators in the trial can be found at:

* Johns Hopkins in Baltimore, MD has a pancreatic cancer screening
study called CAPS4 that is open to PJS participants

* H. Lee Moffitt Cancer Center in Tampa Florida also has a pancreatic
cancer screening study

* Dr. Stratakis continues to study PJS patients in Bethesda, MD, USA

ADDITIONAL RESOURCES
* European laboratories, research studies and information

* Many free, full-text, old articles on PJS are available at

*************

ONLINE SUPPORT GROUPS - INHERITED CANCER SYNDROMES

PJS & Juvenile Polyposis syndrome- updated & revised listserv. The
affected, family, friends, medical professionals and researchers are
welcome to join the free online support group 
problem, join digest mode for one daily email.  Or go nomail to search
the archives as needed. If you'd like to join & need help, please write

Peutz-Jeghers Syndrome Support – Facebook Group

Sindrome de Peutz-Jeghers en Espanol
sindromedepeutzjeghers@googlegroups.com

Peutz-Jeghers syndrome in China

Lynch Syndrome or HNPCC

Gardner syndrome

FAP/Gardner syndrome

Li-Fraumeni

Colon Cancer

Cowden/PTEN

Stephanie Sugars



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